Clinical and socioeconomic determinants of survival in biliary tract adenocarcinomas.

BACKGROUND: Despite advances in detection and treatments, biliary tract cancers continue to have poor survival outcomes. Currently, there is limited data investigating the significance of socioeconomic status, race/ethnicity, and environmental factors in biliary tract cancer survival. AIM: To investigate how socioeconomic status and race/ethnicity are associated with survival. METHODS: Data from the Surveillance, Epidemiology, and End Results database for biliary and gallbladder adenocarcinomas were extracted from 1975 to 2016. Socioeconomic data included smoking, poverty level, education, adjusted household income, and percentage of foreign-born persons and urban population. Survival was calculated with Cox proportional hazards models for death in the 5-year period following diagnosis. RESULTS: Our study included 15883 gallbladder, 11466 intrahepatic biliary, 12869 extrahepatic biliary and 7268 ampulla of Vater adenocarcinoma cases. When analyzing county-specific demographics, patients from counties with higher incomes were associated with higher survival rates [hazard ratio (HR) = 0.97, P <0.05]. Similarly, counties with a higher percentage of patients with a college level education and counties with a higher urban population had higher 5-year survival rates (HR = 0.96, P = 0.002 and HR = 0.97, P = 0.004, respectively). CONCLUSION: Worse survival outcomes were observed in lower income counties while higher income and education level were associated with higher 5-year overall survival among gallbladder and biliary malignancies.

Trends in characteristics, management, and outcomes of patients presenting with gastrointestinal bleeding to emergency departments in the United States from 2006 to 2019.

BACKGROUND: Recent epidemiologic studies of trends in gastrointestinal bleeding (GIB) provided results through 2014 or earlier and assessed only hospitalised patients, excluding patients presenting to emergency departments (EDs) who are not hospitalised. AIMS: To provide the first U.S. nationwide epidemiological evaluation of all patients presenting to EDs with GIB METHODS: We used the Nationwide Emergency Department Sample for 2006-2019 to calculate yearly projected incidence of patients presenting to EDs with primary diagnoses of GIB, categorised by location and aetiology. Outcomes were assessed with multivariable analyses. RESULTS: The age/sex-adjusted incidence for GIB increased from 378.4 to 397.5/100,000 population from 2006 to 2019. Upper gastrointestinal bleeding (UGIB) incidence decreased from 2006 to 2014 (112.3-94.4/100,000) before increasing to 116.2/100,000 by 2019. Lower gastrointestinal bleeding (LGIB) incidence increased from 2006 to 2015 (146.0 to 161.0/100,000) before declining to 150.2/100,000 by 2019. The proportion of cases with one or more comorbidities increased from 27.4% to 35.9% from 2006 to 2019. Multivariable analyses comparing 2019 to 2006 showed increases in ED discharges (odds ratio [OR] = 1.45; 95% confidence interval [CI] = 1.43-1.48) and decreases in red blood cell (RBC) transfusions (OR = 0.62; 0.61-0.63), endoscopies (OR = 0.60; 0.59-0.61), death (OR = 0.51; 0.48-0.54) and length of stay (relative ratio [RR] = 0.81; 0.80-0.82). Inpatient cost decreased from 2012 to 2019 (RR = 0.92; 0.91-0.93). CONCLUSIONS: The incidence of GIB in the U.S. is increasing. UGIB incidence has been increasing since 2014 while LGIB incidence has been decreasing since 2015. Despite a more comorbid population in 2019, case fatality rate, length of stay and costs have decreased. More patients are discharged from the ED and the rate of RBC transfusions has decreased, possibly reflecting changing clinical practice in response to updated guidelines.

NASH and the Gut Microbiome: Implications for New Therapies.

Content available: Author Audio Recording.

Brachytherapy and non-cancer mortality in patients with oral cavity and oropharynx SCCs.

BACKGROUND: Oral cavity and oropharyngeal squamous cell cancers (OC-OPSCC) display high cancer-specific mortality and increased non-cancer mortality. We examined cause of death in patients treated for OC-OPSCC with brachytherapy, chemotherapy, external beam radiation, surgery, or combination of modalities. We hypothesized that brachytherapy does not increase non-cancer mortality comparably with external beam radiation. METHODS: A database was constructed from institutional tumor registry and electronic medical record data from all patients with first OC-OPSCC diagnosis at our institution between 2000 and 2010, excluding patients with a second primary cancer at diagnosis. The primary outcome was association between treatment modality and non-cancer mortality. RESULTS: Of 693 eligible patients, 460 were deceased; 84 from primary malignancy and 96 from a non-primary cancer cause, including 24 with a second primary cancer. 193 patients received brachytherapy. Cox proportional hazards regression was performed on treatment regimen, stratified by AJCC stage, race, and sex. Age, smoking history, and alcohol had HRs for death of 1.05 (p < 0.005), 1.37 (p = 0.106), and 2.24 (p < 0.005), respectively, while brachytherapy had a 0.53 HR (p < 0.005) for death. Non-smoking OPC patients had an 88% 5-year OS, suggesting these were largely HPV-driven cancers. In smoking OPC patients, 5-year OS was 61%. Non-cancer mortality HR of 0.36 for brachytherapy-treated patients. CONCLUSION: We report non-cancer mortality from a cohort of curatively treated OC-OPSCC and show a significant correlation between brachytherapy and non-cancer survival, independent of remission status. The impact of brachytherapy in OPC was strongest in smokers.

Anal adenocarcinoma: case report, literature review and comparative survival analysis.

INTRODUCTION: Anal adenocarcinoma is a rare malignancy with a poor prognosis. METHODS: We present a case of rare anal adenocarcinoma in a patient with normal screening colonoscopy. Using the Surveillance, Epidemiology and End Result database between 2000 and 2016, we performed survival analysis among individuals>20 years old comparing anal and rectal cancers. RESULTS: Survival analysis showed that anal adenocarcinoma is associated with worse outcomes compared with rectal adenocarcinoma and anal squamous cell carcinoma. DISCUSSION: This case and survival data illustrate the importance of prompt investigation of symptoms irrespective of colorectal cancer screening status with careful attention to examination of the anal area.

Fever, pancytopenia, and elevated D-dimer in a 95-year-old woman with ehrlichiosis: a case report.

BACKGROUND: Pancytopenia, fever, and elevated D-dimer are significant clinical findings. The differential diagnosis includes hematological malignancies, severe coronavirus disease 2019 (COVID-19), tick-borne illnesses, and other etiologies. CASE PRESENTATION: We report the case of a 95-year-old woman who presented with high fever (103.6 °F), pancytopenia, and markedly elevated D-dimer (32.21 mg/L; reference range ≤ 0.95 mg/L) in late-autumn during the COVID-19 pandemic at a large academic institution. After remaining persistently febrile, a peripheral blood smear was ordered and revealed parasites consistent with Ehrlichia spp. Doxycycline monotherapy led to symptomatic improvement and resolution of her pancytopenia. During her hospital stay, a computed tomography angiogram of the chest revealed pulmonary emboli, and esophagogastroduodenoscopy uncovered arteriovenous malformations. After appropriate treatment, she was discharged on hospital day 7 and has since done well. CONCLUSIONS: Overall, our case offers a dramatic, unexpected presentation of ehrlichiosis in a nonagenarian. To our knowledge, this is the first report of concurrent ehrlichiosis and pulmonary embolus.

Early identification of patients with acute gastrointestinal bleeding using natural language processing and decision rules.

BACKGROUND AND AIM: Guidelines recommend risk stratification scores in patients presenting with gastrointestinal bleeding (GIB), but such scores are uncommonly employed in practice. Automation and deployment of risk stratification scores in real time within electronic health records (EHRs) would overcome a major impediment. This requires an automated mechanism to accurately identify ("phenotype") patients with GIB at the time of presentation. The goal is to identify patients with acute GIB by developing and evaluating EHR-based phenotyping algorithms for emergency department (ED) patients. METHODS: We specified criteria using structured data elements to create rules for identifying patients and also developed multiple natural language processing (NLP)-based approaches for automated phenotyping of patients, tested them with tenfold cross-validation for 10 iterations (n = 7144) and external validation (n = 2988) and compared them with a standard method to identify patient conditions, the Systematized Nomenclature of Medicine. The gold standard for GIB diagnosis was the independent dual manual review of medical records. The primary outcome was the positive predictive value. RESULTS: A decision rule using GIB-specific terms from ED triage and ED review-of-systems assessment performed better than the Systematized Nomenclature of Medicine on internal validation and external validation (positive predictive value = 85% confidence interval:83%-87% vs 69% confidence interval:66%-72%; P < 0.001). The syntax-based NLP algorithm and Bidirectional Encoder Representation from Transformers neural network-based NLP algorithm had similar performance to the structured-data fields decision rule. CONCLUSIONS: An automated decision rule employing GIB-specific triage and review-of-systems terms can be used to trigger EHR-based deployment of risk stratification models to guide clinical decision making in real time for patients with acute GIB presenting to the ED.

Assessing the Effectiveness of Strategies in US Birth Cohort Screening for Hepatitis C Infection.

Chronic hepatitis C infection in the USA is a highly morbid condition and current guidelines recommend one-time screening among the birth cohort (1945-1965). Understanding strategies to optimize screening can help inform future hepatitis C virus (HCV) screening guidelines. A focused literature search was performed using PubMed and manual abstract review from major hepatology conferences over the past 2 years. The search strategy involved using Medical Subject Headings terms for hepatitis C, screening, birth cohort, baby boomers, and 1945-1965. The review was limited to data from the USA. A total of 327 articles were identified and 36 abstracts were included, with studies published between 2012-2019. Strategies including clinician education, electronic medical record alerts, reflex HCV RNA testing, point-of-care testing, multisite (outpatient, inpatient, emergency department, endoscopy suite) initiatives, direct patient solicitation, and utilization of non-physician providers have increased HCV screening rates. However, broad implementation remains less than optimal. Barriers include lack of patient acceptance to screening and engagement in the HCV care cascade. The Veterans Affairs Healthcare System has achieved higher birth cohort screening rates through an integrated approach requiring high-level engagement by leadership and institutional commitment. Multiple strategies for increasing birth cohort screening have been successful, but overall rates of HCV screening remain low. These strategies can inform public health efforts to implement emerging national recommendations for expansion of HCV screening to all U.S. adults age 18 or older.

Safety of compression therapy for venous ulcer disease in the setting of congestive heart failure.

OBJECTIVES: Compression therapy is the mainstay of treatment for patients with venous ulcer disease. There exists a lack of certainty as to the safety of compression therapy in patients with congestive heart failure. METHODS: A retrospective review of 95 patients with the diagnosis of congestive heart failure (systolic, diastolic, or combined), who underwent compression therapy at the wound care center of a large teaching hospital between January 2013 and June 2019, was performed. Patient outcomes including mortality, weight gain, admission for heart failure decompensation, and requirement for diuretic dose increase were compared to the general congestive heart failure population as well as to the registry data. RESULTS: In the compression cohort, with a mean compression period of 310 days, seven patients (7.3%) were admitted for congestive heart failure exacerbation and six (6.3%) underwent diuretic dosage increase. Two patients (2.1%) died during the compression period. These endpoints were not significantly higher than within the general congestive heart failure population. CONCLUSIONS: Compression therapy appears safe amongst patients with stable congestive heart failure.

Perioperative Outcomes of Lower Extremity Revascularization for Rest Pain and Tissue Loss.

BACKGROUND: Critical limb ischemia (CLI) is the clinical manifestation of severe peripheral artery disease presenting as rest pain (RP) and tissue loss (TL). Most studies compare CLI as a homogenous group with claudication with limited database studies specifically studying these differences. We hypothesize that CLI should be stratified into RP and TL because of significant differences in disease severity, comorbidities, and outcomes. METHODS: The American College of Surgeons National Surgical Quality Improvement Program database from 2012 to 2016 was reviewed. All patients with a postoperative diagnosis of CLI undergoing femoral to popliteal bypass (FPB) with vein or graft were identified. Patients were stratified into cohorts based on International Classification of Disease (ICD)-9 or ICD-10 codes for RP or TL (gangrene or ulcer). Univariate and multivariate analyses were performed to examine 30-day mortality, morbidity, major amputation, and readmission adjusting for demographics, comorbidities, and procedural details. RESULTS: There were 5,304 patients. Compared to RP, patients with TL were older (P < 0.0001) and more likely to be dependent (P < 0.0001). TL patients were also more likely to have diabetes (P < 0.0001), congestive heart failure (P < 0.0001), renal failure (P = 0.004), dialysis (P < 0.0001), history of wound infection (P < 0.0001), and sepsis (P < 0.0001). TL patients had higher American Society of Anesthesiologists class (P < 0.0001), were less likely to be transferred from home (P < 0.0001), and more likely to receive an FPB with vein (P = 0.03). Patients with TL had worse perioperative outcomes compared with RP in terms of pneumonia (P = 0.004), unplanned intubation (P = 0.009), cardiac arrest requiring cardiopulmonary resuscitation (P = 0.003), bleeding requiring transfusions (P < 0.0001), sepsis (P < 0.0001), septic shock (P = 0.02), and reoperation (P < 0.0001). TL was associated with significantly higher 30-day morbidity (P < 0.0001), 30-day mortality (P < 0.0001), major amputation (P = 0.0004), and readmission rates (P = 0.005). Patients with TL compared with those with RP also had longer hospital stays (P < 0.0001) and days between operation to discharge (P < 0.0001). TL was independently associated with increased 30-day morbidity (OR: 1.16 [1.00-1.35]) and major amputation (OR: 2.48 [1.29-4.76]) compared with RP. CONCLUSIONS: Patients with RP and TL have drastic differences that impact perioperative mortality and readmissions. TL is an independent predictor of 30-day morbidity and major amputation. The stratification of CLI into RP and TL can provide insight into variations in outcomes and provide a means to quantify the risks associated with the 2 manifestations of the disease.

Early Colonoscopy Does Not Improve Outcomes of Patients With Lower Gastrointestinal Bleeding: Systematic Review of Randomized Trials.

BACKGROUND & AIMS: Guidelines recommend colonoscopy evaluation within 24 hours of presentation or admission in patients with high-risk or severe acute lower gastrointestinal bleeding (LGIB). Meta-analyses of the timing of colonoscopy have relied primarily on observational studies that had major potential for bias. We performed a systematic review of randomized trials to determine optimal timing of colonoscopy for patients hospitalized with acute LGIB. METHODS: We searched publication databases through July 2019 and abstracts from gastroenterology meetings through November 2019 for randomized trials of patients with acute LGIB or hematochezia. We searched for studies that compared early colonoscopy (within 24 hours) with elective colonoscopy beyond 24 hours and/or other diagnostic tests. Our primary outcome was further bleeding, defined as persistent or recurrent bleeding after index examination. Secondary outcomes included mortality, diagnostic yield (identifying source of bleeding), endoscopic intervention, and any primary hemostatic intervention (endoscopic, surgical, or interventional radiologic). We performed dual independent review, data extraction, and risk of bias assessments. We performed the meta-analysis using a random-effects model. RESULTS: Our final analysis included data from 4 randomized trials. Further bleeding was not decreased among patients who received early vs later, elective colonoscopy (relative risk [RR] for further bleeding with early colonoscopy, 1.57; 95% CI. 0.74-3.31). We did not find significant differences in the secondary outcomes of mortality (RR, 0.93; 95% CI, 0.05-17.21), diagnostic yield (RR, 1.09; 95% CI, 0.99-1.21), endoscopic intervention (RR, 1.53; 95% CI, 0.67-3.48), or any primary hemostatic intervention (RR, 1.33; 95% CI, 0.92-1.92). CONCLUSIONS: In a meta-analysis of randomized trials, we found that colonoscopy within 24 hours does not reduce further bleeding or mortality in patients hospitalized with acute LGIB. Based on these findings, patients hospitalized with acute LGIB do not generally require early colonoscopy.

An Algorithm for the Management of Residual Head and Neck Melanoma In Situ Using Topical Imiquimod: A Pilot Study.

INTRODUCTION: Melanoma in situ (MIS) is an early variant of melanoma in which the disease is limited to the epidermis. Standard therapy is currently surgical excision with at least 5-mm margins. In some instances, there are large or anatomically difficult lesions where complete excision will result in significant esthetic or functional impairment. Our experience has shown a 6-week course to be sufficient in certain patients compared with the suggested 12-week course. In this context, we propose a shortened duration of treatment of positive margins with topical imiquimod and propose an algorithm that results in the least amount of dysfunction for the patient. METHODS: Our inclusion criteria were patients undergoing excision of a lesion found to have postsurgical margins, which were positive for MIS. Patients applied 5% imiquimod 3 to 5 times per week for at least 6 weeks at the site of the positive margin(s). Duration and frequency of treatment were titrated based on response. A reexcision skin biopsy using 1-mm margins was performed after residual inflammation subsided. Our endpoints were recurrence and effectiveness with length of treatment. RESULTS: Sixteen patients met our criteria. All patients were found to have MIS in the margins after surgical excision. Twelve of those lesions were classified as MIS in the initial biopsy; the remaining 4 were either melanoma stage T1A or T1B. Mean follow-up time was 14.3 months, and 1 patient was lost to follow-up. We had 3 patients without response, and of these, 1 was found to have atypical squamous cell carcinoma and the other had a recurrence of MIS. Cure rate after use of topical imiquimod was 93.3% (14/15). DISCUSSION: The proposed algorithm for the treatment of residual disease in head and neck melanoma and MIS using topical imiquimod offers various advantages. This anatomical region is cosmetically sensitive, and the option of using 1 mm versus 5 mm reexcision margins after imiquimod treatment yields comparable cure rates with less tissue excised overall. Our algorithm demonstrates that 6 weeks of active inflammation provides comparable results with the traditional 12-week course. This decreased treatment time reduces patient discomfort and improves patient adherence.

Incisional Negative Pressure Wound Therapy Augments Perfusion and Improves Wound Healing in a Swine Model Pilot Study.

BACKGROUND: A commonly used treatment for open wounds, negative pressure wound therapy (NPWT) has recently been used to optimize wound healing in the setting of surgically closed wounds; however, the specific mechanisms of action by which NPWT may benefit patients after surgery remain unknown. Using a swine wound healing model, the current study investigates angiogenesis as a candidate mechanism. METHODS: Multiple excisional wounds were created on the dorsa of 10 male Yorkshire pigs and closed by primary suture. The closed wounds underwent treatment with either NPWT dressing or control dressings in the absence of negative pressure. Dressings were maintained for 8 days followed by euthanasia of the animal. Scar evaluation of the wounds by photographic analysis was performed, and wounds were analyzed for angiogenesis markers by enzyme-linked immunosorbent assay and immunohistochemistry. RESULTS: Scar evaluation scores were observed to be significantly higher for the NPWT-treated sites compared with the control sites (P < 0.05). The enzyme-linked immunosorbent assay results demonstrated increases for vascular endothelial growth factor (VEGF) staining at the incision site treated with NPWT compared with other treatment groups (P < 0.05). In addition, an approximately 3-fold elevation in VEGF expression was observed at the NPWT-treated sites (2.8% vs. 1%, respectively; P < 0.0001).). However, there was no significant difference in immunohistochemistry staining. CONCLUSIONS: The use of NPWT improves the appearance of wounds and appears to increase VEGF expression after 8 days in the setting of a closed excisional wound model, suggesting that improved angiogenesis is one mechanism by which NPWT optimizes wound healing when applied to closed surgical wound sites.

Do Vertical Soft Tissue and Actual Bony Landmarks Correlate in Le Fort I Orthognathic Surgery?

PURPOSE: Vertical changes in Le Fort I orthognathic surgery are critical to the overall esthetic result. Three-dimensional planning enables vertical measurements from the rendered computed tomographic (CT) scan, but intraoperative points are ascribed partially from soft tissues landmarks. This study compared intraoperative soft tissue vertical measurements with pre- and postoperative CT-based values and attempted to validate intraoperative soft tissue landmarks for vertical positioning. MATERIALS AND METHODS: In this retrospective single-cohort study, the authors examined orthognathic procedures performed by a single surgeon at their institution. Patients were excluded if measurements or pre- and postoperative CT scans were lacking. Demographic information and soft tissue perioperative data were tabulated. Clinical vertical measurements included the left medial canthus to the central incisor, the left medial canthus to the left canine, and the right medial canthus to the right canine. Bone measurements were calculated using pre- and postoperative cone-beam CT scans for the same clinical landmarks. Statistical analysis, including paired Student t test, was performed using SPSS. RESULTS: Forty-two patients were identified (mean age, 23 yr; 57% female). The change in pre- and postoperative measurements was analyzed. There was no significant difference in the absolute value pre- and postoperatively between the 2 modalities (P < .2, .1, .1), but there was a significant difference between bony and soft tissue measurements (P < .01). Subset analysis showed differences in postoperative values between Class II and III cases. CONCLUSIONS: These results show a nonlinear but predictable relation between intraoperative soft tissue (medial canthi and maxillary dentition) and CT-measured bony vertical measurements. Understanding this relation enables effective use of intraoperative measurements to reproducibly achieve the desired bony vertical position and allows adjustments to be made to optimize esthetics.

A 3D Mammometric Comparison of Implant-Based Breast Reconstruction With and Without Acellular Dermal Matrix (ADM).

This retrospective study utilizes 3D imaging and mammometrics to compare implant-based breast reconstruction with and without the use of ADM. Previous studies have suggested improved aesthetic outcomes with the use of ADM, but none have been able to quantify this difference. Images were obtained at early and late time points following the expander-implant exchange procedure. Measurements included the point of maximum projection, the superior, inferior, medial and lateral volumetric distribution, and the distance from the point of maximum projection to the inframammary fold along the breast meridian. The patients' demographic information, implant size, and complication rate between the two cohorts were similar. In the early post-operative period, the patients with ADM demonstrated higher medial pole volume; however, this difference did not persist in the late post-operative period. Patients with ADM demonstrated a small but statistically significant greater point of maximum projection and length of lower pole curvature in comparison with the non-ADM cohort. In summary, the results of this study demonstrate improved mammometric measurements when ADM is used in implant-based breast reconstruction, supporting superior aesthetic outcomes in early and late post-operative time points. LEVEL OF EVIDENCE III: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Use of Indocyanine Green for Sentinel Lymph Node Biopsy: Case Series and Methods Comparison.

INTRODUCTION: Sentinel lymph node biopsy is indicated for patients with biopsy-proven thickness melanoma greater than 1.0 mm. Use of lymphoscintigraphy along with vital blue dyes is the gold standard for identifying sentinel lymph nodes intraoperatively. Indocyanine green (ICG) has recently been used as a method of identifying sentinel lymph nodes. We herein describe a case series of patients who have successfully undergone ICG-assisted sentinel lymph node biopsy for melanoma. We compare 2 imaging systems that are used for ICG-assisted sentinel lymph node biopsy. METHODS: Fourteen patients underwent ICG-assisted sentinel lymph node biopsy for melanoma using the SPY Elite system (Novadaq, Mississigua, Canada) and the Hamamatsu PDE-Neo probe system (Mitaka USA, Park City, Utah). We analyzed costs for 2 systems that utilize ICG for sentinel lymph node biopsies. RESULTS: Intraoperative use of ICG for sentinel lymph node biopsies was successful in correctly identifying sentinel lymph nodes. There was no difference between the Hamamatsu PDE-Neo probe and SPY Elite systems in the ability to detect sentinel lymph nodes; however, the former was associated with a lower operating cost and ease of use compared with the latter. CONCLUSION: ICG-assisted sentinel lymph biopsy using the SPY Elite or the Hamamatsu PDE-Neo probe systems for melanoma are comparable in terms of sentinel node detection. The Neo probe system delivers pertinent clinical data with the advantages of lower cost and ease of operation.

Harvey Cushing Treated the First Known Patient With Carney Complex.

CONTEXT: Carney complex (CNC) is a syndrome characterized by hyperplasia of endocrine organs and may present with clinical features of Cushing syndrome and acromegaly due to functional adrenal and pituitary gland tumors. CNC has been linked to mutations in the regulatory subunit of protein kinase A type I-alpha (PRKAR1A) gene. DESIGN: Tissue samples were taken from the hypothalamus or thalamus or tumors of patients with pituitary adenomas seen and operated on by neurosurgeon Harvey Cushing between 1913 and 1932. Following DNA extraction, sequencing for genes of interest was attempted, including PRKAR1A, AIP, USP8, GNAS1, and GPR101, to explore the possibility that these mutations associated with acromegaly, CNC, and Cushing syndrome have been conserved over time. RESULTS: We report a patient described by Dr. Cushing in 1914 with a clinical presentation and postmortem findings suggestive of CNC. Genetic sequencing of the hypothalamus and pituitary adenoma revealed a germline heterozygous p.Arg74His mutation in the PRKAR1A gene, a codon previously described as mutated in CNC, but with a novel amino acid change. CONCLUSIONS: This patient is, to our knowledge, the first molecularly confirmed individual with CNC. This case demonstrates the power of modern genetics in studying archived tissues and the importance of recording detailed clinical notes in the diagnosis of disease.

Preclinical endoscopic training using a part-task simulator: learning curve assessment and determination of threshold score for advancement to clinical endoscopy.

BACKGROUND: Preclinical simulator training has the potential to decrease endoscopic procedure time and patient discomfort. This study aims to characterize the learning curve of endoscopic novices in a part-task simulator and propose a threshold score for advancement to initial clinical cases. METHODS: Twenty novices with no prior endoscopic experience underwent repeated endoscopic simulator sessions using the part-task simulator. Simulator scores were collected; their inverse was averaged and fit to an exponential curve. The incremental improvement after each session was calculated. Plateau was defined as the session after which incremental improvement in simulator score model was less than 5%. Additionally, all participants filled out questionnaires regarding simulator experience after sessions 1, 5, 10, 15, and 20. A visual analog scale and NASA task load index were used to assess levels of comfort and demand. RESULTS: Twenty novices underwent 400 simulator sessions. Mean simulator scores at sessions 1, 5, 10, 15, and 20 were 78.5 ± 5.95, 176.5 ± 17.7, 275.55 ± 23.56, 347 ± 26.49, and 441.11 ± 38.14. The best fit exponential model was [time/score] = 26.1 × [session #]-0.615; r 2 = 0.99. This corresponded to an incremental improvement in score of 35% after the first session, 22% after the second, 16% after the third and so on. Incremental improvement dropped below 5% after the 12th session corresponding to the predicted score of 265. Simulator training was related to higher comfort maneuvering an endoscope and increased readiness for supervised clinical endoscopy, both plateauing between sessions 10 and 15. Mental demand, physical demand, and frustration levels decreased with increased simulator training. CONCLUSION: Preclinical training using an endoscopic part-task simulator appears to increase comfort level and decrease mental and physical demand associated with endoscopy. Based on a rigorous model, we recommend that novices complete a minimum of 12 training sessions and obtain a simulator score of at least 265 to be best prepared for clinical endoscopy.